Allogeneic Bone Marrow Transplantation Appears to Ameliorate IgA Nephropathy in a Patient with X-linked Thrombocytopenia

被引：10

作者：

Hoshino, Akihiro ^{[1
]}

Shimizu, Masaki ^{[2
]}

Matsukura, Hiroyoshi ^{[3
]}

Sakaki-Nakatsubo, Hisano ^{[1
]}

Nomura, Keiko ^{[1
]}

Miyawaki, Toshio ^{[1
]}

Kanegane, Hirokazu ^{[1
]}

机构：

[1] Toyama Univ, Grad Sch Med & Pharmaceut Sci, Dept Pediat, Toyama 9300194, Japan

[2] Kanazawa Univ, Sch Med, Inst Med Pharmaceut & Hlth Sci, Dept Pediat, Kanazawa, Ishikawa 920, Japan

[3] Saiseikai Toyama Hosp, Dept Pediat, Toyama, Japan

来源：

JOURNAL OF CLINICAL IMMUNOLOGY | 2014年 / 34卷 / 01期

关键词：

Aberrant IgA; bone marrow transplantation; IgA nephropathy; Wiskott-Aldrich syndrome; X-linked thrombocytopenia; WISKOTT-ALDRICH-SYNDROME; SYNDROME PROTEIN; DOWN-REGULATION; WASP GENE; T-CELLS; GLYCOSYLATION; MUTATIONS; BETA-1,3-GALACTOSYLTRANSFERASE; GLOMERULONEPHRITIS; PATHOGENESIS;

D O I：

10.1007/s10875-013-9964-4

中图分类号：

R392 [医学免疫学]; Q939.91 [免疫学];

学科分类号：

100102 ;

摘要：

Wiskott-Aldrich syndrome (WAS) is caused by a mutation in the WAS gene, and it is clinically characterized by the triad of thrombocytopenia, eczema and immunodeficiency. X-linked thrombocytopenia (XLT), which is a clinically mild form of WAS, is also caused by a WAS gene mutation. Patients with WAS/XLT sometimes also have autoimmune diseases such as IgA nephropathy. Progression of IgA nephropathy may lead to chronic renal failure with a poor prognosis. Here, we describe an XLT patient who also had IgA nephropathy. The patient underwent bone marrow transplantation (BMT) because of an associated-lymphoproliferative disorder, and clinical and histological improvement in his IgA nephropathy was observed after BMT. The amount of galactose-deficient IgA in the patient's serum markedly decreased after BMT. Therefore, immunological reconstitution might improve autoimmune diseases in patients with WAS/XLT.

引用

页码：53 / 57

页数：5

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